Naslov
Initial presentation of hereditary angioedema as abdominal pain and ascites in puerperium: a case report

Autori
Šalamon, Lea ; Morović-Vergles, Jadranka

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Skup
I kongres hrvatskih alergologa i kliničkih imunologa

Mjesto i datum
Zagreb, Hrvatska, 05.2009

Vrsta sudjelovanja
Poster

Vrsta recenzije
Domaća recenzija

Ključne riječi
hereditary angioedema; ascites; puerperium

Sažetak
Hereditary angioedema (HAE) is an autosomal dominant inherited condition caused by a quantitative or qualitative deficiency of C1-esterase inhibitor (C1-INH). Type 1 HAE is characterized by a low or unmeasurable level of C1-INH, while in type 2 HAE, C1-INH is normal but dysfunctional. Patients with HAE have recurrent episodes of angioedema, without urticaria or pruritus, which most often affect the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. We describe a first reported case of type 1 HAE in a young woman presenting with recurrent abdominal pain associated with ascites, without any other clinical features of HAE and with initial presentation in puerperium. A 22-year-old woman presented with abdominal colic and nausea 4 weeks after uncomplicated vaginal delivery with a full term, live birth. She was admitted to another hospital where physical examination revealed only a temperature of 37, 5&#186; C. Usual laboratory findings showed slightly elevated C-reactive protein (CRP) 12, 3 mg/l (normal values [n. v. ] < 5, 0 mg/l) and white blood cell count to 13, 0 x 109/l (n. v. 4-10). C4 levels were very low, 0, 02g/l (normal values, 0, 1-0, 4) and C3 levels were normal, 1, 12g/L (n. v. 0, 9-1, 8). Abdominal ultrasonography and MSCT of abdomen revealed only moderate volume free peritoneal fluid. Patient was treated with amoxicilinum and clavulanic acid, abdominal pain reduced and she was discharged with a diagnosis of ascites of undetermined origin and abdominal colics. Two similar episodes occurred during a period of next 5 months for which she was hospitalized in two different hospitals where extensive workout was done, symptoms resolved spontaneously and the cause of the symptoms was not established. Patient came to our observation 6-months after the initial presentation with symptoms of occasional nausea and abdominal discomfort. Physical findings and common laboratory tests were unremarkable except for the elevated CRP 13, 2 mg/l. Abdominal ultrasonography revealed a small volume free peritoneal fluid. C4 level was decreased to 0, 02 g/L (n. v. 0, 1-0, 4)) as well as C1-INH level to 0, 0317 g/l (n. v. 0, 18-0, 39) with normal levels of C1q and C3 which are characteristic for type 1 HAE. She declined prophylactic treatment. The awareness of an atypical form of hereditary angioedema as a possible cause of unexplained and recurrent abdominal pain with ascites is important to initiate early treatment and to prevent redundant hospitalizations.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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